Sister Mary Joseph’s Nodule

 

Defined as neoplastic lesion in the periumbilical region arising as a primary tumor or representing as a site of metastasis from visceral organ malignancies such as from the gastrointestinal tract and the reproductive organs. The overall incidence is 1-3% in general population with malignancies. The primary site of malignancy associated is significantly different in men and women. The most common primary site in men is the stomach followed by the colon and pancreas, whereas in women, the most common site is the ovary followed by endometrium, colorectal, and pancreas.

The prognosis of patients presenting with Sister Mary Joseph’s nodule is generally poor as it is a sign of advanced malignancy. Management of the disease should consider patient preference, the clinical state of the patient, and the etiology of the primary malignancy.

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Porcelain gallbladder (PGB)

Term porcelain gallbladder (PGB) is often used to describe calcification of the gallbladder wall. When infiltrated by extensive calcium deposits, the gallbladder wall can become fragile, brittle and bluish in appearance, resulting in a ‘porcelain’ appearance.

The true incidence of porcelain gallbladder is unknown, but it is reported to be 0.6-0.8%, with a male-to-female ratio of 1:5. Most porcelain gallbladders (90-95%) are associated with gallstone.  Mean age at diagnosis is 32 to 70 years.

Patients with porcelain gallbladder are usually asymptomatic, and the condition is usually found incidentally on plain abdominal radiographs, sonograms, or CT images.

Based on early studies which revealed a high association between porcelain gallbladder and gallbladder adenocarcinoma (22-30% of porcelain gallbladders developing gallbladder adenocarcinoma), cholecystectomy has been routinely performed when a porcelain gallbladder is identified.

More recent studies have cast some doubt on the association, and the risk of gallbladder cancer associated with calcification of the wall may be as low as 5-7%. There is no accepted follow-up interval, but the annual incidence of developing gallbladder cancer is likely to be <1% per year.

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Dupuytren’s contracture

Dupuytren’s contracture is predominantly a myo-fibroblastic disease that affects the palmar and digital fascia of the hand and results in contracture deformities. The most commonly affected digits are the fourth and fifth digits. It is a genetic disorder that often is inherited in an autosomal dominant fashion, but is most frequently seen with a multifactorial etiology. There are a number of factors that are believed to contribute to the development or worsening of this disease.

These include:

• Men are more likely to develop the condition than women.
• People of northern European (English, Irish, Scottish, French, and Dutch) and Scandinavian (Swedish, Norwegian, and Finnish) ancestry are more likely to develop the condition.
• Dupuytren's often runs in families.
• Drinking alcohol may be associated with Dupuytren's.
• Diabetes, HIV, Vascular disease, smoking and seizure disorders are more likely to have Dupuytren's.
• Incidence of the condition increases with age.
  
  
  
  
  
  
  
  
  
  

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Dual-energy x-ray absorptiometry (DEXA)

1. Uses x-rays at two energy levels to determine the bone mineral content.
2. Major role in diagnosis of osteoporosis, the assessment of patients' risk of fracture, and monitoring response to treatment.
3. T-score is a number of standard deviations between the patient’s mean BMD and the mean of the population compared with reference populations matched in gender and race.
4. Z-score is the number of standard deviations above or below the mean of age-matched controls.
5. DEXA could be used to measure bone density at many skeletal sites, two sites are typically measured: the first four vertebrae of the lumbar spine posteroanterior, and the proximal femur (“hip”), including the femoral neck and the trochanteric areas and total hip measurement. Femoral neck and lumbar spine are the gold standard for evaluating osteoporosis, with good accuracy and high precision.
6. All women 65 years and older and men 70 years and older should be screened for asymptomatic osteoporosis.

The World Health Organization (WHO) defines T-scores as:

• Greater than or equal to -1.0: normal
• Less than -1.0 to greater than -2.5: osteopenia
• Less than or equal to -2.5: osteoporosis
• Less than or equal to -2.5 plus fragility fracture: severe osteoporosis

Clinical risk factors included in WHO fracture algorithm

• Age
• Low body mass index
• Prior fracture after age 50
• Parental history of hip fracture
• Current smoking habit
• Current or past use of systemic corticosteroids
• Alcohol intake >2 units daily
• Rheumatoid arthritis 

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Splenectomy

 

• Patients who undergo splenectomy are at increased risk of infections secondary to encapsulated organisms: H Influenzae, Streptococcus pneumoniae & Neisseria meningitidis. 
• Vaccinations against these organisms are highly recommended in patients who have undergone splenectomy. 
• Careful attention must be paid to post-splenectomy patients presenting with febrile illnesses as they may require more aggressive, empiric antibiotic therapy.
• Palpation of spleen ---see below
  
  
  
  

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Niacin Deficiency

Distinctive dark red rash appears on the hands, feet, calves, neck, and face, and the tongue and mouth turn dark red.

Many people with niacin deficiency also have deficiencies of protein, riboflavin (a B vitamin), and vitamin B6.

Pellagra develops only if diet is deficient in niacin & tryptophan (body can convert tryptophan to niacin).

Affects the skin, digestive tract, & brain.

Also develops in:

Hartnup disease (absorption of tryptophan is impaired), & Carcinoid syndrome (tryptophan is not converted to niacin).

Alcoholism & isoniazid can lead to a deficiency of niacin.

The diagnosis of niacin deficiency is based on the diet history and symptoms. Measuring a by-product of niacin in urine can help establish the diagnosis, but this test is not always available. The diagnosis is confirmed if niacin relieves symptoms.

Treatment: Nicotinamide, unlike nicotinic acid, does not cause flushing, itching, burning, or tingling sensations.

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White Blood Cell Scan

 

Indium 111- tagged white blood cell scan is a type of imaging modality used to help identify regions of inflammation and thus infections when other imaging studies are equivocal or contraindicated.

The test is used for diagnostic purposes in the evaluation of prosthetic joint infections, osteomyelitis, vascular graft infections, intra-abdominal infections, abscesses, endocarditis, foot ulcers, infected implanted devices such as central venous catheters, fevers of unknown origin when there is a high probability of infection, and Inflammatory bowel disease.

Sensitivity 60 to 100% and specificity 69 to 92%.

White blood cells are obtained from a blood sample from a patient, are tagged with the radioisotope indium-111, and then re-injected intravenously into the patient. These labeled leukocytes localize to a region of inflammation visible on the whole body or regional nuclear imaging with bone scintigraphy.

Recommended dose for adults is 0.3 to 0.5 mCi

Prior IV antibiotics may produce false negative result.

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Pneumobilia vs Portal venous gas

 

Pneumobilia

Also known as aerobilia. Accumulation of gas in the biliary tree. Seen as linear branching gas within liver most prominent in central large caliber ducts as the flow of bile pushes gas toward the hilum. Gas within the biliary tree tends to be more central, whereas gas within the portal venous system tends to be peripheral (carried along by the blood). Also, biliary gas is anti-dependent, and typically fills the left lobe of the liver.

“Saber sign”: Supine radiographs often demonstrate a sword-shaped lucency in the right paraspinal region representing gas from the common duct and the left hepatic duct. Present in ~50% of patients with pneumobilia.

Portal venous gas

Peripheral small caliber branching gas (lucencies) projected in the liver or vessels coursing towards the Liver (away from the hilum).

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High output Ileostomies

 

• Output >1.5 -2.0 L/24 hours leading dehydration & dys-electrolytemia.
• Occurs in 31% of small bowel stomas.
• Daily output increases with increasing small bowel resection
• Resection of 15-50cm of terminal ileum results in an increase of >300 g/24hr vs with <15cm removed 
• Mature ileostomy put out up to 1200mL/day
• Jejunostomies can put out up to 6 L/day
• Colostomies usually only put out 200-600mL/day 
  
  
  

Normal intestinal fluid transport

• 9 -10 L of fluid passes the ligament of Treitz/ day
• Jejunum absorbs ~ 6 L & Ileum ~ 2.5 L
• Colon absorbs rest but 100 mL excreted in feces daily.

Ostomy at ileocecal valve expected to produce 1-1.5 L of stool output/day

Containing approximately

• 200 mEq of sodium
• 100 mEq of chloride &
• 10 mEq of potassium
  
  
  

In Extensive ileal resection, >100 cm, bile salts loss outpaces hepatic production, leading to bile acid deficiency & steatorrhea

Hypomagnesemia occurs in 78% with a jejunostomy.

Common complications include:

• - Dehydration & AKI
• - Low serum sodium
• - Low urinary sodium
• - Low serum magnesium
• - Loss of Chloride & bicarbonate leading to metabolic acidosis
• - High plasma renin & aldosterone
• - Weight loss / malnutrition
• - Low Vitamin B12 (if > 60-100cm of terminal ileum resected)
  
  
  

Management:

Rehydrate & replace electrolytes

Oral hypotonic fluid is restricted & a glucose-saline solution is sipped.

Medication

• To slow transit (Imodium/Lomotil/opioids) or
• To reduce secretions (omeprazole for gastric acid)
• Octreotide/sandostatin
  
  
  
• GLP-2, enhances gut adaptation, inhibits gastric acid secretion & slow emptying; stimulates intestinal blood flow; increases intestinal barrier function; & enhances nutrient & fluid absorption. 

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Sudden Cardiac Death

 Sudden Cardiac Death

Occurs within one hour of the onset of symptoms.

CAD (most common 80%)

(Icy Idiots Chased Hot Vain Chimps)

1. Ischemic Heart disease (MI)
2. Inherited Channelopathies (QT syndrome)
3. Cardiomyopathies (OH, HCM, Myocarditis)
4. Heart Failure (EF less than 35%)
5. Valve disease (Aortic stenosis)
6. Congenital disease (Tetraology of Fallot)

The proximal cause of SCD in most instances is either ventricular fibrillation (VF) or ventricular tachycardia (VT). However, in a significant minority of cases, asystole or pulseless electrical activity is the initial documented rhythm.

The step to improving outcomes involves the chain of survival:

1. Immediate recognition of cardiac arrest and activation of the emergency response system.
2. Early CPR with an emphasis on chest compressions.
3. Rapid defibrillation.
4. Effective advanced life support; and
5. Integrated post-cardiac arrest care.

References:

1. https://www.ncbi.nlm.nih.gov/books/NBK507854/#:~:text=Sudden%20cardiac%20death%20(SCD)%20is,to%20maintain%20perfusion%20and%20life.
2. https://www.ahajournals.org/doi/full/10.1161/01.cir.98.21.2334

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Beta-Blocker Overdose/toxicity

• Bradycardia & hypotension (most common). 
• Myocardial depression & cardiogenic shock (severe overdoses). 
• Ventricular dysrhythmias (Common with propranolol & acebutolol). 
• Others (mental status change, seizure, hypoglycemia, & bronchospasm). 
• Co-ingestions of CCB, TCA, & neuroleptics, increases mortality. 
• Mostly symptomatic < 2 hrs following ingestion, & nearly all develop symptoms < 6 hrs. 
• Delayed toxicity up to 24 hrs after ingestion (Sustained release meds: metoprolol succinate & sotalol). 
• Sotalol prolongs the QTc interval & can lead to Torsades de Pointes. 
• Carvedilol (associated with edema & toxic epidermal necrolysis). 
• IV lipid emulsion therapy for poisoning involving lipophilic medications (eg, propranolol, metoprolol, labetalol).

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QT/QTc- Interval

• Start of Q-wave to end of the T-wave (time of ventricular depolarization + repolarization). 
• Life threatening risk of prolonged QTc >500ms = Torsades de pointes (TdP).
• Prolonged QT/QTc interval may be a clue to electrolyte disturbances (hypocalcemia or hypokalemia), drug effects (quinidine, procainamide, amiodarone, or sotalol), or myocardial ischemia (usually with prominent T wave inversions). 
• Shortened QT intervals are seen with hypercalcemia and digitalis effect. 
• Each 10-millisecond increase in QTc contributes approx a 5% to 7% additional increase in risk for TdP.
• QTc of 540 milliseconds has a 63% to 97% higher risk of developing TdP than a patient with QTc of 440 milliseconds.

How do you measure it:

• Find a lead with the tallest T wave and count the little boxes from the start of the QRS complex to the point where the T wave comes back down to the isoelectric line. 
• Multiply the number of little boxes by 0.04 seconds. 
• Example if you counted 8 boxes then QT interval is 8 x 0.04 = 0.32 seconds (320 milliseconds).
• QT interval should be less than half the preceding R-R interval (Works for regular rates between 65-90).

Methods of calculating the QTc:

• Bazett formula, QTc = QT / √RR.  
• Fridericia formula (QTc = QT / RR1/3)
• Hodges [QTc = QT + 0.00175 x (HR - 60)]
• Framingham linear regression analysis {QTc = QT + 0.154 x (1 - RR)}
• Karjalainen et al. [QT nomogram] 
• Rautaharju formula, QTc = QT x (120 + HR) / 180

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